Motor Neuron Disease

Question 1

biggest difference between progressive bulbar palsy and pseudobulbar palsy

Answer 1

progressive: LMN lesion
pseudo: UMN

Question 2

progressie & pseudo bulbar palsy age of onset

Answer 2

about 50

Question 3

difference of progressie & pseudo bulbar palsy sx/sx

Answer 3

progressive: weakness & ATROPHY of facial muscles, dysarthria, dysphagia, aspiration, drooling dyspnea
pseudo: weakness & SPASTICITY of facial mm, dysarthria, dysphagia, aspiration, drooling

Question 4

prognosis for progressie & pseudo bulbar palsy

Answer 4

poor, 3 years

- also rare diagnoses

Question 5

PSMA & PLS affects what muscles

Answer 5

spinal musculature & limbs

Question 6

PSMA is a ____ lesion whereas PLS is a ____ lesion

Answer 6

PSMA: LMN

PLS: UMN

Question 7

PSMA onset & freqeuncy

Answer 7

30-60 years

variant of ALS

Question 8

PLS onset & frequency

Answer 8

30-60 years

RARE variant of ALS

Question 9

sx/sx of PSMA: what is happening to the muscles

Answer 9

weak & flaccid muscles of extremities/face
muscle twitching/fasciculations
ataxia
incoordination 
atrophy

Question 10

sx/sx PLS: what is happening to the muscles

Answer 10

weak & SPASTIC muscles of extremities/face
clonus 
\+ babinski 
muscle twitching/fasciculations
ataxia
atrophy (not as severe as PSMA)

Question 11

ALS affects what muscles

Answer 11

bulbar and spinal muscular atrophy

Question 12

what MN affected with ALS

Answer 12

UMN, LMN, or both

Question 13

most common onset of ALS

Answer 13

30-60 yo

Question 14

sx/sx ALS

Answer 14

more variable

• weak muscles of extremities/face
• muscle twitching/fasciculations
• ataxia
• incoordination
• atrophy

Question 15

prognosis of ALS

Answer 15

3-5 years
20% live > 5 years
10% live > 10 years

Question 16

etiology of ALS include mutations oin what

Answer 16

C90RF72
SOD
- multi factorial
- involves cell accumulations, autophagy, and death of SC or bulbar neurons

Question 17

ALS affects what columns

Answer 17

lateral

Question 18

who is most affected by ALS

Answer 18

males > females
40-70
caucasian

Question 19

what muscles are spared with ALS? there is no ___ changes seen

Answer 19

ocular

- no cognitive changes seen

Question 20

etiology of botulism

Answer 20

toxin in food or from an infection leads to impaired CA mediated release of Ach at NMJ

Question 21

botulism affects what area of the nervous system

Answer 21

LMN

NMJ

Question 22

groups most affected by botulism

Answer 22

< 5 yo

anyone eating contaminated food

Question 23

sx/sx of botulism

Answer 23

sudden onset of muscular paralysis including

• double vision
• blurred vision
• drooping eyelids
• slurred speech
• difficulty swallowing
• dry mouth
• muscle weakness

Question 24

Lambert eaton syndrome etiology

Answer 24

AI mediated block of voltage gated Ca channels at NMJ

decrease in # of channels or they become nonfunctional leading to a decrease release in Ach

Question 25

LES affects what part of the nervous system

Answer 25

LMN | NMJ

Question 26

botulism affects what muscles

Answer 26

respiratory, facial, and eyes

Question 27

who is most at risk to get LES

Answer 27

anyone with cancer

Question 28

initial sx/sx of LES

Answer 28

LE & UE weakness difficutly walking double vision blurred vision

Question 29

what things are seen later in LES

Answer 29

``` slurred speech diff swallowing dry mouth constipation urinary urgency ```

Question 30

in LES, strength/power ___ with repeated effort

Answer 30

increase

Question 31

MG etiology

Answer 31

AI dx associated with thymic tumor, RA, lupus, or thyrotoxicosis affects # or function of Ach receptors on NMJ

Question 32

MG affects what area of the nervous system

Answer 32

LMN | NMJ

Question 33

MG most commonly affects who

Answer 33

females > males | middle age, but typically women < 40 and males >60

Question 34

sx/sx of MG

Answer 34

slowly progressive fluctuating course diplopia & ptosis, but pupillary reflex not affected dysarthria, dysphagia, generalized weakness

Question 35

with MG, you see generalized weakness especially with

Answer 35

sustained effort | strength/power decrease with repeated effort

Question 36

how to define probable ALS

Answer 36

1. UMN & LMN signs in 2+ regions | 2. some UMN signs must be rostral to LMN deficits

Question 37

how to define probable lab supported ALS

Answer 37

1. UMN & LMN signs in 1 region or 1. UMN signs in 1 region and LMN signs found by EMG in at least 2 limbs and 2. neuroimaging and electrodiagnostic studies exclude other causes

Question 38

how to define possible ALS

Answer 38

``` 1. UMN & LMN signs in only 1 region or 1. UMN signs alone in 2+ regions or 1. LMN signs rostral to UMN signs ```

Question 39

how to define suspected ALS

Answer 39

1. lower but not upper MN signs in at least 2 regions